{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T14:57:12Z","timestamp":1761058632150},"reference-count":38,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":7315,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1986,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The Indian rubber estate workers in Negri Sembilan, Malaysia, who originated from Orissa in India were found to have a high frequency of Hb S (Joishy SK, Hassan K: Clin Res 28:280, 1980). Unlike the usually severe clinical picture of sickle cell anemia seen in African and American blacks, the clinical picture of the disease in this population was mild and many have reached old age. We studied the leukocyte DNA of 12 patients with sickle cell anemia, ranging in age from 4 to 61 years and 30 sickle cell trait carriers, ranging in age from 7 to 63 years, for the presence of \u03b1\u2010globin gene deletions by gene mapping according to Southern (Southern EM: J Mol Biol 98:503, 1975), using \u03b1\u2010 and \u03b6\u2010globin gene probes obtained by nick translation of the \u03b1\u2010 and \u03b6\u2010globin genes cloned into plasmid. All 12 sickle cell anemia patients were found to have \u03b1\u2010thalassemia<jats:sub>2<\/jats:sub> (\u03b1\u2010thal<jats:sub>2<\/jats:sub>), either in the homozygous or heterozygous condition. Of the Hb S trait carriers, six did not have \u03b1\u2010that<jats:sub>2<\/jats:sub> or or\u2010thal<jats:sub>1<\/jats:sub> and 24 had \u03b1\u2010thal<jats:sub>2<\/jats:sub> (15 heterozygous, 9 homozygous). Seven of these Hb S trait carriers with \u03b1\u2010thal<jats:sub>2<\/jats:sub> had an additional gene abnormality. Five of them had a fast\u2010moving Eco RI fragment 5.6 kb long that hybridized with \u03b6\u2010specific probe but not with \u03b1\u2010specific probe. An unusual DNA pattern of a different type was further found in the other two. Bgl II restriction analysis showed that the \u03b1\u2010thal<jats:sub>2<\/jats:sub> was mostly of the rightward deletion \u03b1\u2010thal<jats:sub>2<\/jats:sub> genotype. None of the sickle cell anemia patients and Hb S trait carriers had deletion type \u03b1\u2010thal<jats:sub>1<\/jats:sub>. The sickle cell anemia patients had very high levels of Hb F and low levels of Hb A<jats:sub>2<\/jats:sub>. The Hb S trait carriers with \u03b1\u2010thal<jats:sub>2<\/jats:sub> had relatively low levels of Hb S.<\/jats:p>","DOI":"10.1002\/ajh.2830220307","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T10:47:33Z","timestamp":1172054853000},"page":"265-274","source":"Crossref","is-referenced-by-count":4,"title":["Sickle cell anemia associated with \u03b1\u2010thalassemia in Malaysian Indians"],"prefix":"10.1002","volume":"22","author":[{"given":"Luan Eng","family":"Lie\u2010Injo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Khalid","family":"Hassan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suresh K.","family":"Joishy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mu Lan","family":"Lim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(72)92592-5"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-88-1-1"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1978.tb05813.x"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1980.tb07163.x"},{"key":"e_1_2_1_6_2","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/S0308-2261(21)00626-3","article-title":"Fetal haemoglobin in homozygous sickle cell disease","volume":"4","author":"Sergeant GR","year":"1975","journal-title":"Clin Haematol"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/61.3.375"},{"key":"e_1_2_1_8_2","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1182\/blood.V22.6.757.757","article-title":"The first observation of homozygous hemoglobins\u2010alpha thalassemia disease and two types of sickle cell thalassemia disease: (a) Sickle\u2010cell\u2010alpha thalassemia disease, (b) sickle\u2010cell\u2010beta thalassemia disease","volume":"22","author":"Aksoy M","year":"1963","journal-title":"Blood"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1969.tb01402.x"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830060202"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198202043060504"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198206173062402"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/j.1834-4461.1962.tb01765.x"},{"key":"e_1_2_1_14_2","first-page":"144","article-title":"Sickle cell anemia in an Indian family in Malaysia","volume":"14","author":"Vella F","year":"1959","journal-title":"Med J Malaya"},{"key":"e_1_2_1_15_2","first-page":"101","article-title":"Hereditary haemolytic anaemias in West Malaysia","volume":"24","author":"Lopez CG","year":"1969","journal-title":"Med J Malaya"},{"key":"e_1_2_1_16_2","first-page":"288\u2010A","article-title":"Sickle cell anemia in Malaysia: Clinical, hematologic and genetic features","volume":"28","author":"Joishy SK","year":"1980","journal-title":"Clin Res"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2836(75)80083-0"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.18.6.790"},{"key":"e_1_2_1_19_2","first-page":"413","article-title":"Studies on abnormal hemoglobins. Their demonstration in sickle cell anemia and other hematologic disorders by means of alkali denaturation","volume":"6","author":"Singer K","year":"1951","journal-title":"Blood"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1038\/251392a0"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830180311"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI109984"},{"key":"e_1_2_1_23_2","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1182\/blood.V59.2.370.370","article-title":"Hb Bart's level in cord blood and deletions of \u03b1\u2010globin genes","volume":"59","author":"Lie\u2010Injo LE","year":"1982","journal-title":"Blood"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1017\/S1120962300017650"},{"key":"e_1_2_1_25_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830060203"},{"key":"e_1_2_1_26_2","doi-asserted-by":"crossref","first-page":"706","DOI":"10.1182\/blood.V55.4.706.706","article-title":"Alpha globin gene number: Population and restriction endonuclease studies","volume":"55","author":"Brittenham G","year":"1980","journal-title":"Blood"},{"key":"e_1_2_1_27_2","first-page":"801","article-title":"Hematologic parameters in obligatory alpha\u2010thalassemia traits","volume":"52","author":"Pornpatkul M","year":"1969","journal-title":"J Med Assoc Thai"},{"key":"e_1_2_1_28_2","first-page":"136","article-title":"The relationship between the serum iron concentration and erythrocytic haemoglobin A2 level","volume":"2","author":"Kuczynski A","year":"1971","journal-title":"J Med"},{"key":"e_1_2_1_29_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V62.2.286.286"},{"key":"e_1_2_1_30_2","doi-asserted-by":"crossref","first-page":"1353","DOI":"10.1182\/blood.V63.6.1353.1353","article-title":"the Cooperative Study of Sickle Cell Disease: Effect of thalassemia and microcytosis on the hematologic and vasooclusive severity of sickle cell anemia","volume":"63","author":"Steinberg MH","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_31_2","doi-asserted-by":"crossref","first-page":"1042","DOI":"10.1182\/blood.V64.5.1042.1042","article-title":"Dense cells in sickle cell anemia: The effect of gene interaction","volume":"64","author":"Fabry ME","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_32_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V65.1.183.bloodjournal651183"},{"key":"e_1_2_1_33_2","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1182\/blood.V64.2.452.452","article-title":"Two independent genetic factors in the \u03b2\u2010globin gene cluster are associated with high G\u03b3\u2010levels in the Hb F of SS patients","volume":"64","author":"Gilman JG","year":"1984","journal-title":"Blood"},{"key":"e_1_2_1_34_2","first-page":"311","article-title":"The coexistence of the genes for haemoglobin E and \u03b1thalassaemia in Thais with resultant suppression of haemoglobin E synthesis","volume":"16","author":"Tuchinda S","year":"1964","journal-title":"Am J Hum Genet"},{"key":"e_1_2_1_35_2","doi-asserted-by":"publisher","DOI":"10.1159\/000207867"},{"key":"e_1_2_1_36_2","doi-asserted-by":"publisher","DOI":"10.1038\/202499a0"},{"key":"e_1_2_1_37_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V21.4.479.479"},{"key":"e_1_2_1_38_2","doi-asserted-by":"publisher","DOI":"10.1016\/0035-9203(66)90183-0"},{"key":"e_1_2_1_39_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.81.6.1771"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.2830220307","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.2830220307","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,21]],"date-time":"2023-10-21T13:18:42Z","timestamp":1697894322000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.2830220307"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1986,7]]},"references-count":38,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1986,7]]}},"alternative-id":["10.1002\/ajh.2830220307"],"URL":"https:\/\/doi.org\/10.1002\/ajh.2830220307","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[1986,7]]}}}